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Article
Growth Pattern and Sexual Maturation Rate in β-Thalassemia Major Patients from Thalassemia Center Erbil

Authors: Nazar Baker --- Abdulkader Alnakashabandi --- Azhar H. Alsaqy --- Abbas Alrabaty
Journal: Iraqi Academic Scientific Journal المجلة العراقية للاختصاصات الطبية ISSN: 16088360 Year: 2013 Volume: 12 Issue: 1 Pages: 40-44
Publisher: The Iraqi Borad for Medical Specialization المجلس العراقي للاختصاصات الطبية

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Abstract

ABSTRACT: BACKGROUND: Thalassemia is genetic disorder in globin chain production, or it refers to a group of blood disease characterized by decrease synthesis of one of two types of poly peptide chain ( α or ß ) that form a normal adult human hemoglobin molecule ( Hb A- α2 ß2 ) resulting in decrease filling of red cell with hemoglobin and anemia .Growth retardation can occur as a complication of thalassemia as early as the 1st or 2nd year of life but these abnormalities are more prominent after the 6 – 8 years of life . OBJECTIVE: The main objective of the present study was to evaluate the relationship of growth failure and sexual maturity rate (SMR) in children with β-thalassemia major in comparison with controls. MATERIAL AND METHOD : In this case-control study, the growth parameters ( height ,weight ,and sexual maturation ) and S.ferritin of 38 patients aged 8-16 years (24 males 14 females) with β-thalassemia major who were attending thalassemia center in Erbil city Iraqi Kurdistan were compared with those of 38 healthy controls of the same age and gender. RESULTS: Underweight and short stature were found in 23 (61%), 30(79%) of patient group and 3(8%), 3(8%) of control group, the mean age of menarche for female patients was 12.31±2.3 and for control female 11.12±1.31years, The SMR were delayed in 37(97.5%) of patients and in 2 (5.5%) of controls. the level of serum ferritin had no significant relationship with delayed SMR. CONCLUSION: Growth failure (underweight and short stature) and delay SMR significantly occur in thalassemia patients compared to controls, and such growth retardation was more likely to occur after 10 years of duration of the disease


Article
The Prevalence of Cases of B-Thalassemia Minor Among the Hematologically Suspected Cases in Kerbala.

Authors: Duha Maitham Hassan1 --- Mohammed Shnain Ali
Journal: Karbala Journal of Medicine مجلة كربلاء الطبية ISSN: 19905483 Year: 2017 Volume: 10 Issue: 3 Pages: 3841-3845
Publisher: Kerbala University جامعة كربلاء

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Abstract

background: B-thalassemia minor is the symptomless carrier state of B-thalassemia that can be misdiagnosed as mild iron deficiency anemia (IDA).Aim: This work aims to assess the prevalence of cases of B-thalassemia minor among the suspected cases and to compare the results of diagnosis depending on single confirmatory test (Hb electrophoresis) and three confirmatory tests (reticulocyte count, serum ferritin and Hb electrophoresis).Materials and methods: In this study 50 cases of mild anemia (Hb ≥9-11.99 g/dl) with initial hematological suspicion of B-thalassemia minor were studied for detailed confirmatory diagnostic tests that include reticulocyte count, Hb electrophoresis for HbA2 estimation and S.Ferritin level.Results: Results showed that 43/50 i.e 86% of suspected cases are confirmed by three tests (41/50 i.e (82%) have B-thalassemia minor, 2/50 i.e (4%) have B-thalassemia minor and IDA), 6/50 i.e (12%) have iron deficiency anemia (IDA), while 1/50 i.e (2%) have polycythemia (on venesections). Discussion: Results showed that 86% of initially suspected cases are confirmed to have B-thalassemia minor and 4% of cases have B-thalassemia minor with IDA depending on three tests for confirmatory diagnosis which are reticulocytosis, HbA2 concentration and S.Ferritin level. Similar results were encountered by Cristina Passarello, Antonino Giambona, et al.Conclusion: The prevalence of cases of B-thalassemia minor among the hematological suspected cases in Kerbala is 86%.


Article
The Influence Of Parathyroid Gland On Beta-Thalassaemia Patients in Kirkuk City
تأثير الغدة جار الدرقية على مرضى بيتا ثلاسيميا في مدينة كركوك

Authors: Demet F. Saber دمت فكرت صابر --- Mossa M. Marbut موسى محمود مربط --- Sami A. Zbaar سامي اكريم زبار
Journal: Tikrit Journal of Pure Science مجلة تكريت للعلوم الصرفة ISSN: 18131662 Year: 2019 Volume: 24 Issue: 2 Pages: 10-13
Publisher: Tikrit University جامعة تكريت

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Abstract

Thalassaemia is an inherited blood disorder in which the body makes an abnormal form of hemoglobin. The symptoms of thalassemia appear in the first two years of life and include paleness of the skin, anemia, bone abnormalities, weakness and growth retardations. (70) male thalassaemic patients are diagnosed in thalassaemia center at Azadi Teaching Hospital in Kirkuk city and( 30) normal male healthy Controls were included in this study Anthropometric measures include, Age, BMI, were done for all participants. there is significant elevation, (P> 0.01) in PTH in P2 age group (9-13) years as compare with control healthy subjectsand as compare with P1(4-8) and P3(14-18) age group

الثلاسيميا هو اضطراب دموي موروث يصنع فيه الجسم شكلاً غير طبيعي من الهيموجلوبين. تظهر أعراض الثلاسيميا في السنتين الأوليين من العمر وتشمل شحوب الجلد وفقر الدم والتشوهات العظمية والضعف وتأخر النمو ولا يوجد حتى الآن أكثر الطرق نجاحاً للشفاء التام لمرض الثلاسيميا. تضمنت الدراسة الحالية (70) سبعين عينة دم لذكور مصابين بالثلاسيميا في مركز الثلاسيميا بمستشفى ازادي التعليمي في مدينة كركوك وفي هذه الدراسة تم تسجيل القياسات الانثروبومترية والعمر ومؤشر محتوى كتلة الجسم لجميع المرضى ومجموعة السيطرة وقد اظهرت نتائج الدراسة الحالية ارتفاع ملحوظ (P> 0.0)في المجموعة العمرية الثانية لمستوى هرمون الغدة الجار الدرقية (9-13) مقارنة مع المجوعة العمرية الاولى (4-8) والمجموعة العمرية الثالثة (14-18).

Keywords

Thalassemia --- PTH --- Calcium --- S.ferritin --- ALP


Article
MRI Assessment of Liver and Cardiac Iron Concentrations in Some Patients with Beta Thalassemia Major

Author: Mohammed Abd kadhim *, Zainab Kassim Al-Jobouri**, Ammar Mosa Jawad***, Mohssin Abd Ali Hussain****
Journal: Iraqi Academic Scientific Journal المجلة العراقية للاختصاصات الطبية ISSN: 16088360 Year: 2018 Volume: 17 Issue: 1 Pages: 82-90
Publisher: The Iraqi Borad for Medical Specialization المجلس العراقي للاختصاصات الطبية

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Abstract

ABSCTRACT:BACKGROUND: Iron overload is a major concern in blood transfusion dependent beta-thalassemic patient and it is a major cause of cardiac dysfunction. Magnetic resonance imaging T2* has a vital role in liver and cardiac iron deposition and assessment of its severityOBJECTIVE:To highlight the role of MRI T2* in assessment of liver and cardiac iron deposition and assessment of other methods of liver and cardiac iron concentration estimation.PATIENTS AND METHODS: This cross sectional prospective study had been conducted at the MRI unit of Al-Imamain AL-Khadimain medical city, Baghdad/ Iraq, from December 2015 to December 2016. One hundred, blood transfusion dependent beta-thalassemia major patients had been examined by MRI using T2* images to assess its value which had been changed to liver iron concentration and cardiac iron concentration by using a MEDIS software program. RESULTS:One hundred patients (56 male and 44 female) of transfusion dependent beta-thalassemia major had been evaluated for their liver and cardiac T2*, 17 of them shows a mild LIC (liver iron concentration) and normal MIC (myocardial iron concentration), 49 patients show mild increase in both LIC and MIC, 9 show moderate LIC and mild MIC, 16 show moderate both LIC and MIC, 2 show severe LIC with mild MIC, only one shows severe LIC with moderate MIC and 6 of them show severe both LIC and MIC. There is a positive significant correlation between liver T2* and cardiac T2* (P values of 0.017), between liver T2* and LIC (P value of > 0.001), between liver T2* and MIC (P value of 0.031) and that between cardiac T2* and MIC (P value is >0.001). There is non- significant correlation between the age and frequency of blood transfusion with LIC and MIC (P value <0.05). There is also a significant negative correlation between S.ferritin and liver T2*and cardiac T2* (correlation coefficient of -0.251 and -0.397 respectively), with a (P value of 0.014 and 0.00015 respectively). There is non significant correlation between S. ferritin and the severity of LIC and MIC (P value of 0.129 and 0.792). CONCLUSION: Magnetic resonance imaging has a vital role as a non-invasive and reliable method in the assessment of liver and cardiac iron deposition in patients with blood transfusion dependent beta-thalassemia major. There is no correlation between S.ferritin level and the severity of LIC and MIC. Patients using Exjade show a lower LIC and MIC than those using Desferal. No correlation between age and frequency of blood transfusion with LIC and MIC in patients on regular chelation. *.


Article
Study of Some Endocrine Glands Dysfunction and Their Effects on Growth in Beta- Thalassemia Major Patients in Thiqar Governorate

Authors: Moaed E. Al-Gazally --- kadhum j. alhamdani --- Naeem Salih Yaser
Journal: Journal of University of Babylon مجلة جامعة بابل ISSN: 19920652 23128135 Year: 2015 Volume: 23 Issue: 2 Pages: 626-640
Publisher: Babylon University جامعة بابل

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Abstract

Background :Beta-thalassemia is a herediatory haemoglobinopathy and because of severity of anemia in this disease, the patients require regular blood transfusion, large number of complications occure from the disease itself or from its treatment like blood transfusion or chelating therapy. Aims of study : study of thyroid dysfunction , growth hormone deficiency,short stature and diabetes mellitus in patient with beta-thalassemia major in Thiqar governorate.Patients and Mthods: this study include 80 beta-thassemia major patients aged 2-18 years and 80 subjects were included as control group.Morning venous blood samples were withdrawn from all participants. T3, T4,TSH and S.ferritin were estimated by ELFA technique while GH was estimated by ELISA technique. S.glucose and Hb were estmited by colorimetric methods . Results:High significant decrease in blood Hb levels in thalassemic patients as compare to control subgroups (p<0.01).High significant increase in S.ferritin levels in thalassemic patient as compare to control subgroups (p<0.01).The percentage of diabetes mellitus was 3.75%( 3/80) and all above ten years. Percentage of GH deficiency was 23.7% (19/80) and present in all ages and short stature prevalence was 36/80 (45%) . GH deficient form 50% of short stature patients . Primary hypothyroidism forms (2/80) 2.5% of total patients and male to female ratio was 1:1 and both were more than ten years old age .There was significant elevation in S.ferritin in GH deficienct as compare to other patients (p<0.05). No statistical correlation between S.ferritin and thyroid hormones .Conclusions : Variation in Hb levels between patients reflect the difference in severity of thalassemic disease which may be due to genetic causes.GH deficiency and short stature are present in all ages.The difference in S.ferritin levels among patients associate with accumulated blood transfusion and compliance with chelation therapy

خلفية الدراسة : مرض فقر الدم البحري الكبير نوع بيتا هو من امراض اختلال خضاب الدم الوراثية وبسبب فقر الدم الحاد المصاحب للمرض يحتاج المرضى الى نقل الدم وبصورة متكررة مما يؤدي الى الكثير من المضاعفات نتيجة المرض نفسة او نتيجة العلاج مثل نقل الدم او ادوية ازالة الحديد .اهداف الدراسة: هو دراسة اختلال وظائف الغدة الدرقية, هرمون النمو, قصر القامة, وداء السكري بين مرضى فقر الدم البحري الكبير نوع بيتا في محافظة ذي قار .المرض والطرق : تضمنت الدراسة الحالية 80 مريضا من المصابين بمرض فقر الدم البحري الكبير نوع بيتا, حيث تراوحت أعمارهم بين 2- 18 سنة , أما مجموعة السيطرة فقد تضمنت 80 شخصا وقد قسمت كل من المجموعتين , الى مجموعتين فرعيتين عمريتين , تراوحت الأعمار في المجموعة الفرعية الأولى من 2- 9 سنوات , أما في المجموعة الفرعية الثانية فتراوحت الاعمار بين 10- 18 سنة . تم جمع عينات الدم باستخدام تقنية التألق المرتبط بالانزيم وفرتين مصل الدم TSH بالاضافة الى T4,T3. حيث تم قياس هرمونات الغدة الدرقية. للأشخاص في الصباح اما مستوى السكر في الدم وخضاب الدم , ELISA بـاستخـدام تقنية الأمـتـزاز المناعي المرتبط بالانزيم بينما تم قياس هــرمون الـنمــو. ELFA فقد تم قيــاس كـل منهمــا باستخدام الطريقــة اللـونيــة . النتائج : تبين من نتائج الدراسة الحالية وجود ارتفاعا معنويا عاليا في مستوى فرتين مصل الـدم في مجموعـتي المرضى مقارنة بمجموعتي السيطرة (0.01(p< وكذلك ارتفاعا معنويا في المجمـوعـة العمرية الثانية للمرضى مقارنة بالأولى (0.05(p< كما لـوحـظ أن هنـاك انخفاضا معنـويا عـاليـا في مستوى خضاب الدم في مجموعتي المرضى مقارنة بمجموعتي السيطرة (0.01(p<, ولم يتبين وجود فروقا معنوية بين المجموعة الأولى والثانية للمرضى.كما وجد ثلاث حالات من مرضى داء السكر 3 /80 , وكانت نسبة الذكور للإناث 2: 1وكانت اعمارهم اكثر من عشرة اعوام . كانت نسبة نقص هرمون النمو بين كل المرضى 19/80 وبنسبة مئوية 23.7% وتبين وجود نقص هذا الهرمون لدى جميع الفئات العمرية قيد الدراسة , وكانت نسبة الذكور للإناث 10: 9, أما نسبة قصر القامة فكانت36 /80, وبنسبة مئوية 45% وكان 50% منهم مصاب بنقص هرمون النمو .كما تبين ان نسبة المصابين بانخفاض افراز الغدة الدرقية الابتدائي هي 2 /80 وبنسبة مئوية 2.5% وكانت نسبة الذكور للاناث1 : 1وكلا المريضين كانت اعمارهم اكثر من عشرة اعوام وقد تبين من نتائج هذة الدراسة وجود ارتفاع معنوي في مستوى فرتين مصل الدم للمرضى المصابين بنقص هرمون النمو مقارنة ببقية المرضى (0.05(p< بينما لم تتضح وجود علاقة احصائية بين مستوى الفريتين ومستوى هرمونات الغدة الدرقية . الاستنتاجات: الاختلافات في مستويات خضاب الدم للمرضى تعكس الاختلافات في حدة مرض الثلاسيميا والتي يمكن ان تعزى لاسباب جينية ان نقص هرمون النمو وقصر القامة موجود في كل الفئات العمرية.الاختلافات في مستويات فرتين مصل الدم للمرضى يتعلق بالكمية المتراكمة من الدم المعطى وكذلك الالتزام بتناول الادوية المضادة للحديد.

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